Spectrum of childhood Epstein-Barr virus-associated T-cell proliferations and bone marrow findings.
Pediatr Dev Pathol
; 14(1): 28-37, 2011.
Article
em En
| MEDLINE
| ID: mdl-20367453
ABSTRACT
Systemic Epstein-Barr virus-positive (EBV+) T-cell lymphoproliferative disorder of childhood is a recently described entity. The majority of such cases have been reported from Asia, which suggests an underlying genetic predisposition. We analyzed the clinicopathologic characteristics of 5 children with EBV+ T-cell lymphoid proliferations evaluated and treated at our institute over a 2-year period. There were 3 males and 2 females of Latino (n â=â 4) or Caucasian (n â=â 1) heritage with a median age of 5 years (age range 2-18 years). All patients presented with EBV infection (acute, n â=â 4) with elevated serum EBV viral loads at the time of diagnosis and had systemic manifestations, including fever, hepatosplenomegaly, and pancytopenia. The bone marrow biopsies showed EBV+/CD8+ T-cell lymphocytosis in all patients, with variable degrees of histiocytosis, plasmacytosis, and hemophagocytosis. Interestingly, there was marked and consistent depletion of mature and precursor B cells in the marrow (<1% of total marrow cellularity) in all patients. Three of the patients died of disease-associated complications 2 to 12 weeks after initial diagnosis. Our study describes the detailed bone marrow findings, contributes to the growing number of cases of systemic EBV+ T-cell lymphoproliferative disorder of childhood occurring in the Western hemisphere, and documents this disorder in patients from the Caribbean countries.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Medula Óssea
/
Linfoma de Células T
/
Infecções por Vírus Epstein-Barr
Tipo de estudo:
Diagnostic_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Child, preschool
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
Pediatr Dev Pathol
Assunto da revista:
PATOLOGIA
/
PEDIATRIA
Ano de publicação:
2011
Tipo de documento:
Article
País de afiliação:
Estados Unidos